Ventricular septal defect (VSD) is usually diagnosed in childhood. In adults, it is less often diagnosed due to spontaneous closure of some VSD's during the early years of childhood. Perimembranous VSD is a defect seen in the upper part of the septum and near the valves occurring in nearly 75% of patients. Although spontaneous closure has been reported in VSD, with about 30%–40% closing before 2 years of age and the remainder of cases closing by about 4 years of age, it is rare for VSD's to close after 8 years of age. Most of the small VSD's will remain asymptomatic, but one of the long-term complications of a small VSD is the development of infective endocarditis (IE). Most of the studies in adults with small VSD's and IE had many complications such as arrhythmias, subaortic and sub-pulmonic stenosis, and exercise intolerance. Here, we report a patient with small perimembranous VSD who was asymptomatic in childhood but suddenly become symptomatic due to development of IE. In this patient, the vegetations were large, multiple chunky linear vegetations partly sealing the VSD defect and also occluding the right ventricular outflow tract and pulmonary valve mimicking severe pulmonary stenosis, which was managed conservatively.